Face recognition is a primary social skill which
depends on a distributed neural network. A pronounced
face recognition deficit in the absence of any lesion is seen
in congenital prosopagnosia. This study investigating 24
congenital prosopagnosic subjects and 25 control subjects
aims at elucidating its neural basis with fMRI and voxelbased
morphometry. We found a comprehensive behavioral
pattern, an impairment in visual recognition for faces
and buildings that spared long-term memory for faces with
negative valence. Anatomical analysis revealed diminished
gray matter density in the bilateral lingual gyrus, the right
middle temporal gyrus, and the dorsolateral prefrontal
cortex. In most of these areas, gray matter density correlated
with memory success. Decreased functional activation
was found in the left fusiform gyrus, a crucial area for
face processing, and in the dorsolateral prefrontal cortex,
whereas activation of the medial prefrontal cortex was
enhanced. Hence, our data lend strength to the hypothesis
that congenital prosopagnosia is explained by network
dysfunction and suggest that anatomic curtailing of visual
processing in the lingual gyrus plays a substantial role. The
dysfunctional circuitry further encompasses the fusiform
gyrus and the dorsolateral prefrontal cortex, which may
contribute to their difficulties in long-term memory for
complex visual information. Despite their deficits in face
identity recognition, processing of emotion related information
is preserved and possibly mediated by the medial
prefrontal cortex. Congenital prosopagnosia may, therefore,
be a blueprint of differential curtailing in networks of
visual cognition.